A clinicopathologic and immunohistochemical review was made of seven cases of angiomyofibroblastoma. The patients were middle-aged women who had a slowly growing mass, measuring 1.5-6 cm in maximum dimension, located subcutaneously in the vulva. The tumors were well-demarcated and characterized by well-vascularized, alternating hypercellular and hypocellular edematous areas composed of bland, plump spindle- or oval-shaped stromal cells frequently aggregated around small blood vessels. An epithelioid appearance of the stromal cells was seen in two cases. Immunohistochemically, the stromal cells were consistently positive for vimentin and desmin, but negative for muscle specific actin, alpha-smooth muscle actin, myosin, cytokeratins, S-100 protein or von Willebrand factor. Ultrastructurally, the plump stromal cells had a small amount of peripherally located rough endoplasmic reticulum, numerous pinocytotic vesicles and abundant intermediate filaments, on which immunogold probes for desmin were localized, whereas fine filaments were few and there were no electron dense plaques. Thus, while the proliferating stromal cells expressed an immunohistochemical profile of peculiar myoid differentiation, ultrastructural findings differed from those of smooth muscle cells or those seen in typical myofibroblasts. At 1-4 years after surgery, there was no evidence of recurrence.

A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb.

These results suggest that a subgroup of desmoid tumours at risk of recurrence may be hypervascular lesions associated with trisomy 8.

Two cases are reported of a rare association of intramuscular myxoma with fibrous dysplasia in a 70 and 40 year old Japanese woman, respectively. One of them had a solitary intramuscular myxoma, and the other patient suffered from two intramuscular tumors that had been initially misdiagnosed as myxoid liposarcoma. Only 24 cases of this kind of association have been recorded in the literature. This association should be taken into consideration to avoid inappropriate treatment, when cases of myxoid soft tissue tumor with a bone lesion are encountered.

The case of a 49-year-old man with Maffucci's syndrome, who developed multiple spindle cell hemangioendotheliomas, is presented. The case provides support for recent reports suggesting an association between this peculiar vascular lesion and skeletal enchondromatosis.